tbuckley/cabot-search huggingface dataset:
AB1504 CLINICAL SPECTRUM OF IGG4-RELATED DISEASE. SINGLE UNIVERSITY HOSPITAL EXPERIENCE AND LITERATURE REVIEW
Authors: L. Sanchez-Bilbao, J. Loricera, C. Álvarez-Reguera, A. Herrero-Morant, R. Blanco
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> IgG4-related disease (IgG4-RD) is an inflammatory and fibrosing entity with very heterogeneous clinical manifestations. Its pathogenesis remains unknown, clinical features are heterogeneous and unspecific, and recently released classification criteria are invaluable in early recognition of the disease. Therefore, regrettably IgG4 related disease continues underdiagnosed. <h3>Objectives</h3> <b>a</b>) To evaluate the clinical characteristics of patients diagnosed with IgG4-RD in a single University hospital; <b>b</b>) To compare with other large series. <h3>Methods</h3> Study of patients from a referral hospital and literature review of cases diagnosed with IgG4-RD. Diagnosis was made accordingly to these criteria<b>: a</b>) Okazaki; <b>b</b>) Umehara; <b>c</b>) ACR/EULAR 2020; and/or <b>d</b>) clinical, laboratory and imaging suggestive findings <b>(ref. 1-3</b>). For the literature review, we searched PubMed and the Cochrane library from its inception until 31 December 2022, selecting the largest series. <h3>Results</h3> We include 11 patients (7 females/4 males) (mean±SD age; 61.2±15.6 years) diagnosed with IgG4-RD. The organs affected at diagnosis were: aorta (n=5), pleura/lung (n=4), lymph nodes (n=3), salivary glands (n=2), retroperitoneum (n=2), lacrimal glands (n=1), bile duct (n=1), kidney (n=1), orbit (n=1), subglottis (n=1), pericardium (n=1), mesentery (n=1), maxillary sinuses (n=1). IgG4 values were increase in 2 (18%) patients (median [IQR]; 250.5 [201.0-300.1] mg/dL) (normal value &lt;135 mg/dL). Blood plasmablasts were increased in 8 (73%) patients (median; 831 [785-1218] cells/mL) (normal values &lt;653 cells/mL). In the literature review, 6 series of more than 100 patients each were selected. The main data from the different series are listed in <b>table 1</b>. The <b>figure</b> shows the most frequently affected organs in the different series. The pancreas was one of the most frequently involved. In contrast, in our series, aortic involvement followed by lung/pleura were the most frequent. <h3>Conclusion</h3> IgG4-RD is a very heterogeneous disease with involvement of virtually every organ of the anatomy, usually presenting with involvement of more than one organ. Despite the name of the entity, serum IgG4 is not always elevated. <h3>References</h3> [1]Okazaki K et al. Int J Rheumatol. 2012. PMID: 22690221 [2]Umehara H, et al. Mod Rheumatol. 2012. PMID: 21881964 [3]Wallace ZS et al. Arthritis Rheumatol. 2020. PMID: 31793250 <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> Lara Sanchez-Bilbao: None declared, Javier Loricera Speakers bureau: Roche, Novartis, UCB Pharma, MSD, Celgene, and Grünenthal, Carmen Álvarez-Reguera: None declared, Alba Herrero-Morant: None declared, Ricardo Blanco Speakers bureau: AbbVie, Pfizer, Roche, Bristol-Myers, Lilly, Janssen, and MSD, Grant/research support from: AbbVie, MSD, and Roche.
AB1065 BERTOLOTTI SYNDROME: AN UNDER RECOGNIZED CAUSE OF INFLAMMATORY BACK PAIN?
Authors: S. Stebbings, C. White, T. Doyle
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Bertolotti syndrome describes a lumbosacral transitional vertebra (LSTV) which causes symptoms, usually low back pain. LATV is a congenital anomaly where the L5 vertebra has an unusual morphology. Bertolotti syndrome is an under-recognised condition by clinicians. <h3>Objectives</h3> To clarify the presentation of Bertolotti syndrome, whether there are features of inflammatory back pain (IBP) and the effect on quality of life. <h3>Methods</h3> In this pilot study, 62 patients with LSTV were identified on imaging (plain x-ray). Imaging was performed for a variety of indications, predominently for back pain. In total, 34 patients agreed to take part, with 18 returning questionnaires. Questionnaires were selected for face vailidity and included: Calin IBP Questionnaire, EQ-5D Questionnaire, Visual analogue pain scale (VAS-P). In view of Covid restrictions all contact was by phone and questionnaires were completed online or returned via post. Plain X-rays visualising the lumbar spine were assessed for radiological features of LSTV. <h3>Results</h3> Seventeen (94%) of the participants (n=18) recorded a VAS-P score &gt;3, indicating a clinically significant level of pain. The mean VAS-P score was 6 (range of 2-9). 89% of respondents scored at least 3/5 in the Calin questionnaire. Of the 5 features of inflammatory back pain in the Calin questionnaire, 4 out of the 5 were reported by most respondents. The exception was 'improvement on exercise', which was only reported by 18% of respondents. Quality of life was impaired-EQ-5D (mean: 0.503, range -0.074 to 0.796). The commonest radiological abnormality was enlarged transverse process (100%) followed by pseudoarticulation with the sacrum (83%) and scoliosis (33%). Presence of sclerosis and/or osteoarthritis at the pseudoarticulation was associated with worse pain scores. Female respondents reported worse pain. <h3>Conclusion</h3> These results suggest Bertolotti syndrome is associated with pain in the majority of patients and affects quality of life,. The character and site of the pain suggests that Bertollotti syndrome should be considered in the differential diagnosis of spondyloarthritis. <h3>REFERENCES:</h3> NIL. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> Simon Stebbings Consultant of: Abbvie. Janssen, Ciara White: None declared, Terence Doyle: None declared.
AB1631 ASSOCIATION BETWEEN HYPERURICEMIA AND HEARING IMPAIRMENT: RESULTS FROM THE KOREAN NATIONAL HEALTH AND NUTRITION EXAMINATION SURVEY
Authors: H. Jeong, Y. S. Chang, E. J. Park, C. H. Jeon
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Hyperuricemia is associated with variety of comorbidities. Researchers suggest that the inflammation and increased oxidative stress produced by uric acid can impact the auditory system. <h3>Objectives</h3> This study investigated the association between hyperuricemia and hearing impairment in Korean adults. <h3>Methods</h3> Audiometric and laboratory test data from the 2019 to 2020 Korean National Health and Nutrition Examination Survey (KNHANES) were used for analysis. Hearing impairment was defined as a pure tone average (0.5,1,2,4 kHz) threshold level of ≥ 41 desibels (dB)s. Definition of hyperuricemia was different in males and females (&gt;7 mg/dL in males and &gt;6 mg/dL in females. <h3>Results</h3> A total of 4,857 (weight n = 17,990,725) subjects were analyzed. Mean age was 56.8 years old and female was 51.7%. Weighted prevalence of hyperuricemia was 12.1%. and hearing impairment was 13.4%. In univariable analysis, hyperuricemia was significantly associated with hearing impairment, but diagnosis of gout was not associated with hearing impairment. In multivariable analysis, hyperuricemia (Odds ratios [OR] 1.41, 95% confidence interval [CI] 1.03-1.92, p = 0.030) was associated with hearing impairment along with age (OR 1.12, 95% CI 1.10-1.14, p &lt; 0.001), female (OR 0.43, 95% CI 0.34-0.64, p &lt; 0.001), education (OR 0.43, 95% CI 0.30-0.63, p = 0.001), and occupational noise exposure (OR 1.67, 95% CI 1.25-2.22, p = 0.001). In subgroup analysis, hyperuricemia was associated with hearing impairment in female group and elderly group aged 60 years or older (OR 1.59, 95% CI 1.02-2.48, p = 0.041 and OR 1.45, 95% CI 1.05-1.99, p = 0.023, respectively). <h3>Conclusion</h3> Hyperuricemia was independently associated with hearing impairment especially in female and older ages. <h3>References</h3> [1]Singh JA, Cleveland JD. Gout and hearing impairment in the elderly: a retrospective cohort study using the US Medicare claims data. <i>BMJ Open</i>. Aug 20 2018;8(8):e022854. doi:10.1136/bmjopen-2018-022854 [2]Moon IJ, Byun H, Woo SY, et al. Factors Associated With Age-related Hearing Impairment: A Retrospective Cohort Study. <i>Medicine (Baltimore</i>). Oct 2015;94(43):e1846. doi:10.1097/md.0000000000001846 <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
AB0131 ASSOCIATION OF CYTOKINE AND CLINICAL EVALUATION BASED ON ULTRASOUND OF SALIVARY GLAND GRADE CLASSIFICATION IN PATIENTS WITH EARLY PRIMARY SJÖGREN’S SYNDROME
Authors: Y. F. Chen, Y. F. Fang
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Primary Sjögren's syndrome (pSS) is a chronic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands, the presence of anti-SSA/Ro, anti-SSB/La antibody and hypergammaglobulinemia. <h3>Objectives</h3> To investigate the clinical manifestations in patients with early primary Sjögren's syndrome (pSS), and cytokine difference based on the severity score under salivary gland ultrasonography. <h3>Methods</h3> Forty-six newly diagnosed patients with early pSS were enrolled and divided into mild (score 0-1) and severe (score 2-3) groups according to the salivary gland ultrasonography grade (SGUS) scores at baseline. Clinical evaluation, ESSPRI and ESSDAI index values, sicca symptoms of mouth, salivary capacity, and serum autoantibody and cytokines were investigated. <h3>Results</h3> Mean age of pSS patients at diagnosis were 50.02 ± 12.4 years and the mean duration of sicca symptoms were 1.4 years. ESSPRI (EULAR Sjögren's syndrome patient report index) and ESSDAI (EULAR Sjögren's syndrome disease index) scores were 5.71 and 5.37, respectively. The higher prevalence of rheumatoid factor (<i>p</i>=0.04), antinuclear antibody (<i>p</i>=0.009) and elevation of total IgG (<i>p</i>=0.08) were found in severe group than in mild group. In addition, elevated titer of IL-18, IL-31 and IL-33 were detected in patients with pSS than in healthy subjects, but there were shown no statistical significance between mild and severe groups. <h3>Conclusion</h3> Salivary gland ultrasonography grade (SGUS) scans may help physician diagnose pSS. Clinical manifestation including the low production of saliva and autoantibody production such antinuclear antibodies, rheumatoid factor, and anti-SSA antibody were found. The elevated titer of IL-18, IL-31 and IL-33 in patients may be implicated in the pathogenesis of pSS. <h3>References: NIL.</h3> <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
AB1428 PSORIATIC ARTHRITIS: CLINICAL FEATURES IN CHILDHOOD
Authors: S. Dahmani, N. Zhuravleva, T. Smirnova, V. Diomidova, A. Arkhipova
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Diagnosis and treatment of psoriatic arthritis require attention from specialists. Clinical manifestations of psoriatic arthritis in children are variable. <h3>Objectives</h3> The purpose of the study: to evaluate the clinical picture of psoriatic arthritis (PsA) in children. <h3>Methods</h3> The case histories of 23 children with psoriatic arthritis aged 3 to 17 years who were treated by a rheumatologist from 2019 to 2022 were analyzed. <h3>Results</h3> Probable psoriatic arthritis was diagnosed in 7 (29%) patients, definite psoriatic arthritis was diagnosed in 16 (71%) patients. At the same time, 12 (53%) children fell ill at the age of up to 7.5 years. In 7 (29%) patients, the disease began with skin lesions, in 16 (71%) patients immediately with articular syndrome. In the group of patients with certain psoriasis, plaque psoriasis was noted, it was detected in 17 (76.3%) children, guttate psoriasis was in 3 (13.5%), isolated psoriasis of the nails in 3 (10.2%) children. In 5 (21.7%) children, lesions of the skin and nail plates were observed. It was noted that psoriatic onychodystrophy was represented by onycholysis, an "oil spot" in the area of fingers and toes in 5 (23%) patients, and thimble symptom was diagnosed in 4 (18.1%) patients. At the onset of the disease, oligoarticular or asymmetric articular syndrome was noted in 65.6% of cases, a symmetrical rheumatoid-like variant in 20.2% of cases, and psoriatic spondylitis in 14.2% of cases. The dynamics of the articular syndrome after 3 years of observation and treatment indicated the development of symmetrical rheumatoid-like arthritis in 41.2% of patients, asymmetric oligoarthritis in 24.1% of children, and spondyloarthritis in 22.1%. <h3>Conclusion</h3> The clinical picture of psoriatic arthritis in children is variable. Skin lesions preceded the development of arthritis in children. At the onset of the disease, asymmetric oligoarthritis predominated, followed by transformation into symmetrical rheumatoid-like arthritis. <h3>REFERENCES:</h3> NIL. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
AB0983 THE DIFFERENCE IN DISEASE ACTIVITY SCORES AND TREATMENT RESPONSE ACCORDING TO GENDER IN PATIENTS WITH AXIAL SPONDYLOARTHRITIS
Authors: J. Lee, S. Y. Kang, S. Lee, H. Kim, E. M. Koh, H. S. Cha
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Measurement of disease activity of axial spondyloarthritis (axSpA) is mainly dependent on the patient reporting outcomes. It is known that there are differences in patient reporting outcomes between men and women. <h3>Objectives</h3> We investigated the differences in disease activity scores before and after treatment with biologic disease-modifying antirheumatic drugs (bDMARDs) between gender. <h3>Methods</h3> Data were retrieved from the Korean College of Rheumatology Biologics therapy (KOBIO) registry. axSpA patients from December 2012 to August 2021 were included in the study. Bath ankylosing spondylitis disease activity index (BASDAI) and ankylosing spondylitis disease activity score (ASDAS) at the first visit after starting bDMARDs were compared between male and female patients. Generalized linear model analyses were conducted to check the relationship between the change in disease activity scores and gender with the correction of other clinical variables. <h3>Results</h3> In total, 1,753 patients (1,343 male and 410 female patients) were included in the analyses. At baseline, male patients were younger, had longer disease duration, and had a higher ratio of HLA-B27 positive patients, higher ratio of radiographic disease, lower BASDAI, and higher CRP level. At the first follow-up visit, male patients achieved BASDAI &lt; 4 more significantly than female patients (79.57% vs. 71.54%, p &lt; 0.0010). However, the proportion of patients who achieved ASDAS low disease activity (&lt; 2.1) and ASDAS inactive disease (&lt;1.3) were not significantly different between gender (71.14% vs. 71.5%, p = 0.8892; 38.82% vs. 39.44%, p = 0.8265, respectively). The difference in BASDAI between the first follow-up visit and baseline was statistically significantly related to gender after adjusting for other clinical information at baseline (p = 0.0033). In the same analysis, the difference in ASDAS was not significantly related to gender (p = 0.1303). <h3>Conclusion</h3> Depending on the method of calculating disease activity, the interpretation of treatment response of bDMARDs may differ between gender. <h3>Acknowledgements</h3> This work was supported by a National Research Foundation of Korea (NRF) grant funded by the Korean government (MSIT) (No. 2021M3H9A2097957 and no. 2021R1F1A1062148). The funders had no role in the study design; in the collection, analyses, or interpretation of data; in the writing of the manuscript; or in the decision to publish the results. <h3>Disclosure of Interests</h3> None Declared.
POS1178 IDENTIFYING THE NEW DISORDER “IDIOPATHIC HYPOCRYOGLOBULINAEMIA” IN PATIENTS WITH PREVIOUSLY UNIDENTIFIED CLINICAL CONDITIONS
Authors: D. Roccatello, S. Sciascia, C. Naretto, A. Barreca, L. Solfietti, L. Battaglia, L. Viziello, R. Fenoglio, D. Rossi
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> A considerable number of patients with high clinical suspicion for cryoglobulinaemic vasculitis either. show negative results for the detection of cryoglobulins or show only trace amounts which cannot. be characterized for composition. <h3>Objectives</h3> We aimed at establishing whether the failure to detect or the detection of trace amounts of cryoglobulin with conventional methods either identifes a peculiar subset of low level cryoglobulinaemia (from now on hypocryoglobulinaemia) or represents a separate entity. <h3>Methods</h3> Using a modifed precipitation technique in hypo-ionic medium, we prospectively identified between 2008 and 2021 237 patients (median age 60.8 years [22–97], 137 females) having &lt; 0.5% cryocrit and clinical suspicion of autoimmune disorder. <h3>Results</h3> Of these 237 patients, only 54 (22.7%) had a history of HCV infection. One hundred and sixty-nine out of 237 patients (71%) had an established underlying disease, while 68 patients (28.6%) (median age 62.9 years [29–93], 35 females) did not show either laboratory markers or clinical symptoms consonant with an underlying aetiology. These 68 cases with only trace amounts of cryoglobulins were defned as having a putatively idiopathic hypocryoglobulinaemia. Nineteen of these 68 patients (27.9%) had a history of HCV infection. Twentyfour patients out of 68 (35.3%) were positive for rheumatoid factor (RF), while 25 (36.7%) patients had signs of complement consumption (i.e., C4&lt; 15 mg/dl and/or C3 &lt; 80 mg/dl), and 36 (52.9%) had increased infammatory indexes. Seven patients only had arthralgia and constitutional symptoms while 61 out of 68 (89.7%) presented with at least one of the three cardinal signs of cryoglobulinaemic vasculitis including skin lesions, peripheral nerve involvement, and glomerulonephritis. Seventy-fve percent of the subjects had type III hypocryoglobulins. In patients with hypocryoglobulinaemia the histologic features of glomerulonephritis (also examined by electron microscopy) resembled those of mixed cryoglobulinaemia-associated glomerulonephritis. <h3>Conclusion</h3> In conclusion, hypocryoglobulins are often polyclonal and are mainly unrelated to HCV infection. Patients who present high clinical suspicion for vasculitis, especially glomerulonephritis and yet test negative for cryoglobulinaemia detected by standard techniques, could require deeper investigation even in the absence of HCV infection, RF activity or signs of complement consumption. <h3>Reference</h3> [1]Meltzer, M. et al. Cryoglobulinemia, a clinical and laboratory study. Cryoglobulins with rheumatoid factor activity. Am. J. Med. 40, 837–856 (1966). <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
POS1432 CAR-TREGS FOR SYSTEMIC LUPUS ERYTHEMATOSUS
Authors: M. Doglio, A. Ugolini, B. Camisa, C. Toma, R. Norata, S. Del Rosso, F. Ciceri, F. Sanvito, M. Casucci, A. Manfredi, C. Bonini
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Systemic Lupus Erythematosus (SLE) is an autoimmune disease characterized by an abnormal inflammatory response against nuclear antigens with consequent tissue damage. Autoreactive B cells and auto-antibodies have a fundamental role in SLE pathogenesis. Regulatory T cells (Tregs) physiologically maintain the immune tolerance and are impaired in SLE. Polyclonal Treg trnasfer obtained unsatisfactory results due to the low number of disease-relevant antigen-specific cells. Chimeric Antigen Receptors (CARs) are molecules capable of redirecting T cell specificity. CAR-Tregs proved effective in pre-clinical mouse models of autoimmunity. <h3>Objectives</h3> We aimed at developing a CAR-Treg based product to be employed in SLE. <h3>Methods</h3> We isolated Tregs from Healthy Donors Peripheral Blood Mononuclear Cells (PBMCs) and expanded them with IL-2 and rapamycin. We transduced Tregs with a Lentiviral Vector encoding for a second-generation anti-CD19 CAR, considering the relevant role of autoreactive B cells and autoantibodies in SLE. <h3>Results</h3> Engineered cells retained their immune suppressive capabilities upon polyclonal stimulation. Noticeably, they acquired new antigen-specific suppressive capacities, being able to block autologous B cell proliferation. We set up a humanized mouse model of SLE. In vivo, CAR-Tregs delayed the occurrence of B cell lymphopenia, producing immunomodulatory cytokines and without showing toxicity or reprogramming towards Th17 pro-inflammatory cells. In inflamed organs, CAR-Tregs restored the normal composition of the immune system. <h3>Conclusion</h3> In conclusion, we efficiently generated anti-CD19 CAR-Tregs and proved their efficacy both in vitro and in an in vivo humanized mouse model of lupus. <h3>REFERENCES:</h3> NIL. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
AB0434 RHEUMATOID FACTOR AS PREDICTOR OF RESPONSE TO RITUXIMAB
Authors: B. Touil, N. Akasbi, I. El Mezouar, T. Harzy
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> In Rheumatoid Arthritis (RA), the presence of rheumatoid factor (RF) indicate poor prognosis. Strategies searching biomarkers that predict response to biologic therapies allowing the selection of patients with the highest chance for response are pertinent. Whether RF is a biomarker of response to RTX is disputed. [1] <h3>Objectives</h3> To investigate whether high rheumatoid factor (3 times normal) is predictive of a good therapeutic response in RA patients treated with Rituximab (RTX). <h3>Methods</h3> We conducted a Retrospective study in a single rheumatology hospital in Morocco. All patients satisfied the ACR-EULAR 2010 RA classification criteria. Socio-demographic data, comorbidities, disease-related variables (duration, characteristics, and drugs) were collected. The endpoint was a decrease in DAS28 greater than 1.2 at 6 month after RTX therapy. Statistical analysis were performed with SPSS 20. <h3>Results</h3> We included 129 patients with a mean age of 58.45 ± 11.99 years. The sex ratio F/M was 8:1. The mean duration of RA was 17.73 ± 8.34 years. The mean age at diagnosis was 44.13 ± 13.91 years, 87% of patients were seropositive. The positivity of rheumatoid factor and ACPA was in 82% and 69% of the patients respectively. RF was highly elevated in 48.4% of patients. In bivariate analysis, strong RF positivity was significantly associated with a good response as assessed by DAS28 (p=0.002), and to a high initial IgM level (p= 0.018). <h3>Conclusion</h3> In our study, RA patients with strong positive rheumatoid factor had a better therapeutic response as assessed by DAS28. These results should be taken into account when making therapeutic choices. <h3>Reference</h3> [1] Maneiro RJ, Salgado E, Carmona L, Gomez-Reino JJ. Rheumatoid factor as predictor of response to abatacept, rituximab and tocilizumab in rheumatoid arthritis: Systematic review and meta-analysis. Semin Arthritis Rheum. 2013 Aug;43(1):9-17. doi: 10.1016/j.semarthrit.2012.11.007. Epub 2013 Jan 2. PMID: 23290690. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.